ABSTRACT
Objetivo: Describir la evolución clínica postquirúrgica de una serie de casos de pacientes con Miastenia Gravis juvenil (MGJ) tratados con timectomía por toraoscópica videoasistida (TVA) derecha. Materiales y Métodos: Estudio retrospectivo que incluyó 13 pacientes pediátricos con diagnóstico de MGJ sometidos a timectomía toracoscópica derecha en la Unidad Médica de Alta Especialidad Hospital de Pediatría, Centro Médico Nacional Siglo XXI de México, entre marzo de 2016 y abril de 2022. Los pacientes fueron caracterizados clínicamente y la enfermedad fue clasificada de acuerdo a los criterios de Osserman. La evolución postquirúrgica se evaluó con la clasificación de DeFilippi para determinar la proporción de pacientes con mejoría y la remisión completa. Resultados: Los pacientes incluidos fueron, en su mayoría, mujeres (84,6%) con edad promedio al diagnóstico fue de 11,1 ± 3,1 años. Las cuatro clasificaciones de MG fueron incluidas, con mayor proporción de MG generalizada leve (38,5%), seguida de ocular (23,1%) y generalizada moderada grave (23,1%). La evaluación de la progresión postquirúrgica demostró que a los tres meses de seguimiento 92,3% presentó mejorías, incluyendo la disminución del uso de medicamentos. La remisión total solo se registró en uno de los pacientes. Los pacientes que tuvieron cirugía antes de los 12 meses de evolución de la MGJ presentaron mejores resultados post timectomía por TVA. Conclusión: Se demostró la utilidad de timectomía por TVA en pacientes pediátricos mexicanos con MGJ. Nuestra experiencia agrega evidencia de que los pacientes pediátricos se benefician de la timectomía, mejorando su estado clínico y disminuyendo el uso de medicamentos y complicaciones e la enfermedad.
Objective: To describe the post-surgical clinical evolution of a case series of patients with juvenile myasthenia gravis (JMG) treated with right video-assisted thoracoscopic (TVA) thymectomy. Materials and Methods: Retrospective study that included 13 pediatric patients with JMG who underwent right TVA thymectomy at the Siglo XXI National Medical Center of Mexico between March 2016 and April 2022. Patients were clinically characterized, and the disease was classified according to Osserman's criteria. Post-surgical evolution was evaluated using the DeFilippi classification to determine the proportion of patients with improvement and complete remission. Results: The included patients were mostly women (84.6%) with a mean age at diagnosis of 11.1 ± 3.1 years. The four MG classifications were included, with the highest proportion of mild generalized MG (38.5%), followed by ocular (23.1%) and moderate-severe generalized (23.1%). The evaluation of post-surgical progression showed that after three months of follow-up, 92.3% presented improvements, including a decrease in the use of medications. Complete remission was only recorded in one of the patients. Patients who underwent surgery before 12 months of evolution of JMG had better results after TVA thymectomy. Conclusion: The usefulness of TVA thymectomy in Mexican pediatric patients with JMG was demonstrated. Our experience adds evidence that pediatric patients benefit from thymectomy by improving their clinical status and decreasing the use of medications and complications of the disease.
ABSTRACT
BACKGROUND: Juvenile myasthenia gravis (JMG) is an antibody mediated autoimmune disorder that manifests as progressive voluntary muscle weakness and fatigue. In medically refractory cases, thymectomy has been shown to abrogate symptoms and reduce glucocorticoid dependence. While transcervical or transsternal incisions have been the traditional approach, adult trends now favor thoracoscopic thymectomy. Little data exist to support this approach in children. METHODS: A retrospective review of all patients younger than 20â¯years of age who underwent a thymectomy for JMG at two pediatric institutions between 2001 and 2018 was performed. Children were divided into either an open (transcervical or transsternal) or thoracoscopic group and baseline characteristics, perioperative, and postoperative outcomes were compared. RESULTS: Thirty-four thymectomies were performed during the 18-year study period; 18 via an open and 16 via a thoracoscopic approach. The operative time was shorter for open procedures compared thoracoscopic ones (108⯱â¯49 and 145⯱â¯43â¯min, respectively, pâ¯=â¯0.025). Thoracoscopic thymectomy was associated with less intraoperative blood loss (5.5⯱â¯6.0 vs 55⯱â¯67â¯ml, pâ¯=â¯0.007), decreased duration of postoperative intravenous narcotic use (5.0⯱â¯1.5 vs 20⯱â¯23â¯h, pâ¯=â¯0.018), and a shorter length of hospitalization (1.7⯱â¯1.0 vs 2.7⯱â¯1.1â¯days, pâ¯=â¯0.009). No perioperative complication occurred in either group. Clinical improvement was reported in 94% of children in both groups. CONCLUSIONS: Thoracoscopic thymectomy in children is a safe and effective surgical technique for the treatment of JMG. Increased acceptance of this minimally invasive approach by children, families, and referring neurologists may enable earlier surgical intervention. TYPE OF STUDY: Clinical research paper. LEVEL OF EVIDENCE: III.